There remain undoubtably many sections of the country with sizable black populations where very little medical care is available. In these and other more fortunate areas virtually nothing is known approximating the true incidence of sickle cell disease nor its morbidity or mortality. Funds must be provided to support development of such studies and to provide some care, education, and counseling of victims so pinpointed.

In terms of realistic figures of funds required to support a fairly circumscribed screening and education program, the following are illustrative: Establishment of a proposed pilot project in Davidson County, Tenn., which anticipates, among other things, utilizing a mobile screening unit to initially screen 24,000 black schoolchildren. It has been estimated this project would cost a minimum of a paltry sum of $50,000 in direct costs. In subsequent years, this program would be expanded to include other locales in the region as far as the southern parts of Kentucky and northern parts of Alabama and possibly also into some areas of Mississippi where Meharry Medical College currently has various programs in operation. This type of program is planned to utilize community organizations and recruit volunteer workers.

Using currently accepted incidence figures, this program in 5 years should screen approximately 150,000 to 175,000 people. As a result, some 2,500 patients with hemoglobin S will be picked up per year. Of this group of victims, approximately 57 to 60 or so per year would actually have sickle cell disease. The economic impact of this is illustrated by the fact that when you consider an average which may be fairly minimal of five crises per year requiring hospitalization, this would cost approximately $19,000 to $20,000 a year for this small number of patients. This is based on an estimate of about 4 to 5 hospital days at an average figure for hospitals across the country set by the American Hospital Association at $78 per day per patient.

The figures which we have presented are a fairly reasonable estimate based on data collected from various reported medical studies and our current hospital figures.

No estimate has been attempted in this statement on such things as outpatient costs, which certainly should be contained in the legislation proposed.

In summary, for all too long, sickle cell anemia has been virtually ignored as an important disease among a major segment of this Nation's population. The victim, maligned and used in many instances. as only a "good source of interesting research material." The time certainly has come to give adequate support to such programs as will provide both care for the victim and continue to expand our knowledge of this patient and his disease. It is hoped that the current spurt of interest in sickle cell disease will remain high and provide the victim with support so long denied.

(The prepared statement of Dr. Rhodes follows:)

TEXT OF TESTIMONY OF ROBERT S. RHODES, M.D. - ON S 2676

To Select Senate Subcommittee

Chairman Edward L. Kennedy

12 November 1971

Senator Kennedy, my name is Dr. Robert S. Rhodes. I am an Assistant Professor of Medicine in Hematology at Meharry Medical College in Nashville, Tennessee. I also hold an appointment on the Medical Faculty at Vanderbilt University and I am a Josiah Macy Foundation Faculty Fellow. As a hematologist I am involved with sickle cell disease from both the research and clinical points of view.

Sickle cell disease is a heritable disorder with red cells containing an abnormal hemoglobin called "Hemoglobin S, Hemoglobin "S" when present is either the symptomatic dreaded, homozygous form (SS sickle cell anemia) or heterogygous

Hemoglobin S in the United States occurs, in about 8-11% of the blacks in the United States. Of those carrying it about 2% have the anemic form of the disease. In other words, approximately one black child in every 400 born in the United States will have sickle cell anemia.

The sickle cell trait, the heterozygous form, can have many of the deadly manifestations of sickle cell anemia as we shall point out. In most instances however, this is compatible with a normal life span and is discovered most often during incidental hemoglobin electrophoresis or family studies.

There are usually no deviations from the normal with respect to physical examination, and except that an occassional patient's urine specific gravity will not

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exceed 1.010, the usual laboratory determinations are normal.

Occassionally, a

patient with sickle cell trait has a vaso-occlusive phenomenon, or a hemolytic process when unique anatomic or functional conditions exist.

Recent observations in the clinic and at the postmortem table indicate that extensive in-vivo sickling can be fatal in sickle cell trait if extreme hypoxia or intravascular pooling with stasis takes place. Such conditions have been seen in marked respiratory depression (deep anesthesia, acute alcholism) or in spinal anesthesia with dilitation of the splanchnic vasculature.

Hematuria usually spontaneous but occassionally following trauma, urinary tract infection or hypotensive episodes occurs at a significant frequency. It may occur with or without pain or clots, it may be prolonged and recurrent and is usually from the left kidney.

Sickle cell trait, under conditions that bring about low oxygen

tension, can result in serious and potentially fatal clinical complications. These complications can be avoided or "minimized" if the physician is aware of existing latent abnormality.

Nearly the complete spectrum of the major vaso-occlusive complications seen in sickle cell anemia has been described for the trait with no other pathogenic explanation. These include aseptic necrosis of the bone, intestinal infarction, infarctive necrosis of the liver, pulmonary infarction, neurologic disorder, kidney infarcts, leg ulcer, priapsm and increased complications of pregnancy. These findings all remain a constant threat to young blacks with sickle cell disease, particularly in the patient with manifest symptoms.

Due to the high complement of fetal hemoglobin present in the red cells at birth and the consequent low percentage of hemoglobin S the occurrence of the sickling phenomenon and clinical expression is hindered.

Therefore, sickle cell

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anemia is detected only after the first several months of life.

Sickle cell disease has often been fatal before adolescence, but with

nearly detection, medical management, education and counseling, survival to forty years and longer is now possible. It has been shown that the adolescent ages may show a significant degree of growth failure, delay in appearance of secondary sex characteristics and delay in the onset of menarche.

Once the disease has been established it pursues a rather constant course combining the signs, symptoms, and limitations of persistent anemia with the episodic and unpredictable intercurrences of various types of crises.

Early detection, patient education, follow-up studies and some preventive measures will lessen the severity of the anemia, painful crises and the numerous other complications and morbid features of this disease.

One of the most serious complications is the aplastic aregenerative crisis. It is usually associated with a viral or bacterial infection, the bone marrow is physiologically hypoplastic and ceases to function as an effective blood making organ while the infection persists. The rapid fall in hemoglobin may reach fatal levels in a few days. However, it should be emphasized that the painful crises inless accompanied by a prolonged febrile course are not usually associated with increased severity of the anemia, and that aplastic or aregenerative crisis need not be accompanied by a painful episode.

The laboratory findings are those of severe anemia:

hemoglobin content

usually 5-9 gm/100 mg, with proportional drop in the hematocrit and red cell count resulting in normocytic and normochromic red cell indices. The stained peripheral blood smear shows variation in size and shape of red cells with 0.5 to more than 20% of red cells sickled.

As many of the facts and figures available atest, sickle cell anemia is a

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much studied and written about disease in the medical sphere. In spite of this it continues to be a much neglected disease and a major economic problem in this nation. This malady afflicts countless black people, most of whom are already of marginal economic means and the occurrence of this disease is sufficient to result in impoverishment by frequent expensive hospitalization.

Sickle cell anemia

has been estimated by the World Health Organization to kill over 80,000 children annually world wide, (W.H.O., 1966).

In the past very little money has been made available for work in sickle cell disease. Almost none of this money has been available for patient care, screening, education and counselling.

The development of support programs for sickle cell disease will of natural necessity require adequate expenditures for development of strong victim oriented clinical programs as well as continued research efforts.

I would remind the committee that much of the effort directed toward the

afflicted patient victim of sickle cell disease has been largely experimental. There have been no acolades for the numerous physicians and the several medical centers which have attempted to carry on the clinical efforts against this disease. Adequate funds must be made available to allow upgrading and expansion of existing programs in patient care, screening and population education and counselling. Support must be provided for new inovative approaches in these same areas.

In the past, grants from the national institute of health have given sickle cell disease a low priority in support, when compared with several exceedingly rare disorders (JAMA Vol. 214: 731). The attitude in many quarters has been formulated

Virtually no concern has

by the basic research accomplishments mentioned earlier. been shown for the major problem with this disease: The unfortunate black victim. There remain undoubtably many sections of the country with sizable black populations where very little medical care is available. In these and other more

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